Volume 1

The haemolytic anaemias, congenital and acquired / by J.V. Dacie.

  • Dacie, John V. (John Vivian), Sir.
Date:
1960-1967
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: The haemolytic anaemias, congenital and acquired / by J.V. Dacie. Source: Wellcome Collection.

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    Heilmeyer and his co-workers (1958) reported similar studies on a 34-year-old man, and his brother, also suffering from a hypochromic anaemia refractory to treatment. Siderotic granules in the erythroblasts were unusually large and numerous. The absence of evidence of increased haemolysis, absence of target cells, and the normal mean cell diameter were thought to differentiate the disorder from thalassaemia. However, neither patient was particularly anaemic (haemoglobins, 10-4 and 10-8 g. per 100 ml., respectively). Gelpi and Ende (1958) described two brothers of Swiss-English stock, aged 33 and 39 years, respectively. Their mother and a sister may have had the disorder in a mild form. The more severely affected of the patients was submitted to splenectomy. Benefit was short-lived and subsequently he suffered from recurrent phlebitis. The platelet count ranged between 2 to 3 millions per cu. mm. There was some evidence of excess haemolysis: the 51 Cr half-time of the patient’s cells was 19 \ days (that of normal cells in the patient was 35 days). The life-span of the brother’s erythrocytes was normal (he was less anaemic, haemoglobin, 10 g. per 100 ini.). Serial fiver biopsies in the more severely affected patient demonstrated the progressive development of hsemochromatosis and ultimately glucose tolerance tests gave diabetic-type curves. As mentioned in the first edition of this book (Dacie, 1954, p. 130), the author has made some observations on a family living in London who almost certainly suffer from the same type of “sex-linked anaemia” as is described in the reports mentioned above. Pre-splenectomy and post-splenectomy blood films of the most severely affected patient (a young man), and of his mother, are illustrated in Figs. 94—96. Since then another patient (also a young man) has been studied, who presented with an identical blood picture (after splenectomy). In many respects the above disorder simulates Mediterranean anaemia. However, there are some differences of which the most important is clearly the mode of inheritance (the gene being dominant but only achieving full expression in males). If this mode of inheritance can be confirmed in other families, the separation of “sex-linked anaemia” from Mediterranean anaemia can be considered as proved. The blood pictures of the two conditions are similar if not identical. However, it is possible that the number and size of siderotic granules in developing erythroblasts may prove to be greater in “sex-linked anaemia” than in Mediterranean anaemia (cf. Figs. 92 and 97). Further study on this point is required. Another possibly distinguishing point is the relatively low or even normal levels of Hb-F in “sex- linked anaemia.” (It can, however, be argued that low levels of Hb-F may also be found in clinically severe thalassaemia minor, with comparable degrees of anaemia.) Not only is the marrow iron greatly increased in “sex-linked anaemia” but there is also a general tendency to the deposition of iron throughout the body, particularly in the liver. It does not